Case presentation: We present the case of a 58-year-old woman with a giant retroperitoneal liposarcoma. The patient presented at our clinic with diffuse abdominal pain and enormous abdominal distension. She had swellings and redness of the right leg. CT scan revealed a 50 × 25 cm sized enhancing soft mass. Ultrasound guided biopsy was negative for the presence of malignant cells. A double "J" ureteral stent was placed in the right ureter. We performed surgery with complete resection of the tumorous mass without multiorgan resection and with macroscopic free margins. A well differentiated, lipoma-like subtype of retroperitoneal liposarcoma, weighing 13.4 kg, was diagnosed in the histopathological report. At 6 months after surgery a new CT scan was done and there was no evidence of recurrence. Currently, after 12 months of follow-up, the patient is asymptomatic and disease free. Conclusion: Surgery is the gold standard for treatment of retroperitoneal liposarcomas, well differentiated retroperitoneal liposarcomas, that have a minimal metastatic potential. It is preferable to place a ureteral stent before surgery for minimizing the risk of intraoperative lesions of ureters. Symptoms in lower extremities, such as swelling, redness and others, that are due to compression in the retroperitoneum by giant tumours, can disappear after their surgical removal.